Mitochondria

Question 1

• Endosymbiont hypothesis?
• 4 parts of the mitochondria? Permeability of membranes? Most mito proteins encoded where? How do they enter? (2)
• Fusion? Dependent on? 2 related proteins?
• Fission? Dependent on? 2 related proteins?
• Electron transport: Electrons released from? Transferred to? Occurs where? By? Protons pumped from ___ to ___ to create what? What is generated? Why? How is energy stored? (2)

Answer 1

• Inner mito. membrane bacteria and outer membrane from eukaryotic cell
• Inner membrane, outer membrane, matrix, cristae; outer = semi; inner = less; nucleus
  TOM = Translocase of outer membrane by passive transport
  TIM = Translocase of inner; ATP dependent gated channel with HSP70 to help fold
• Mixing up bad and good for repair; GTP-ase; MFN and OPA1
• Mitophagy, GTP-ase; Fis1 and DRP
• NADH; O2 to form H2O; inner mito membrane; 4 proteins; inner membrane to matrix to create H+ gradient; electric potential; more negative in matrix; electric potential and proton concentration

Question 2

• ATP production: Steps? (3)
• Cell death stimulated with? (2 examples)
• Quality control: Damaged DNA produce? 3 ways this is ensured not to happen?
• Diseases: Mutation to mito fusion machinery? (2) Mutation in mAAA protease? Arsenic inhibits?

Answer 2

• 1.) 3 protons in to make 1 ATP; eneters via ATP synthase 3a and 3B hexamer (F1=Enzyme F0=Channel) 2.) ADP + Pi to ATP coupled with electron transfer 3.) ATP out via ATP-ADP antiporter
• Iscemic injury; necrosis
• Reactive oxygen (ROS); apoptosis; fusion/fission; mito proteases/LAN recognize and degrade misfolded proteins
• AD optic atrophy, CMT; hereditary spastic paraplegia; oxidative phosphorylation